-
British Journal of Cancer Jan 2015Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children and young adults. Several recent studies have shed new light on the alterations in signalling... (Review)
Review
Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children and young adults. Several recent studies have shed new light on the alterations in signalling pathways and the downstream effects of these pathway alterations in RMS. Many of these effects converge on the fibroblast growth factor and insulin-like growth-factor pathways. These new findings improve the current understanding of RMS, thus offering novel potential therapeutic targets and strategies that may improve the outcome for patients with RMS.
Topics: Animals; Cell Cycle; Gene Expression Regulation, Neoplastic; Humans; Intercellular Signaling Peptides and Proteins; Muscle Neoplasms; Oncogenes; Rhabdomyosarcoma; Signal Transduction; T-Box Domain Proteins
PubMed: 25211658
DOI: 10.1038/bjc.2014.471 -
BMJ Case Reports Jul 2021This is the case of a parameningeal alveolar rhabdomyosarcoma (ARMS) in a 13-year-old boy who presented with painless loss of vision in the right eye, but very few other...
This is the case of a parameningeal alveolar rhabdomyosarcoma (ARMS) in a 13-year-old boy who presented with painless loss of vision in the right eye, but very few other physical signs. The ARMS diagnosis was confirmed with imaging and molecular characterisation of the tumour. Despite tolerating the initial chemotherapy and radiotherapy regimens, there was leptomeningeal recurrence and the patient unfortunately passed away. Parameningeal ARMS occurs in an area of the body, which leads to a wide variety of possible presenting symptoms, creating a long list of differentials that can delay treatment. This tumour subtype has a poor prognosis, and due to the location of the tumour around vital structures in the head, treatment toxicities must be taken into account. This highlights the necessity for having a strong index of suspicion for this tumour in atypical presentations in children, and the necessity for prompt treatment to prevent leptomeningeal disease from occurring.
Topics: Adolescent; Child; Family; Head and Neck Neoplasms; Humans; Male; Meningeal Neoplasms; Neoplasm Recurrence, Local; Rhabdomyosarcoma; Rhabdomyosarcoma, Alveolar; Rhabdomyosarcoma, Embryonal
PubMed: 34266827
DOI: 10.1136/bcr-2021-243267 -
The Oncologist Aug 2005Rhabdomyosarcoma is a highly malignant, small blue cell tumor characterized by muscle differentiation. With modern treatment, more than 70% of children and adolescents... (Review)
Review
Rhabdomyosarcoma is a highly malignant, small blue cell tumor characterized by muscle differentiation. With modern treatment, more than 70% of children and adolescents with this disease are cured. Adequate biopsy to obtain sufficient tissue for accurate diagnosis and molecular characterization is critical. Patients must be assessed for tumor extent; the Intergroup Rhabdomyosarcoma Study (IRS) clinical group and Staging system is universally applied in North America. Multidisciplinary therapy is necessary to maximize cure rates. Local control relies on complete surgical excision when possible; those whose tumors are not completely excised and those with alveolar histology tumors require local irradiation to maximize local control. In North America, vincristine (Oncovin); Eli Lilly and Company, Indianapolis, http://www.lilly.com), dactinomycin (Cosmegen); Merck & Co., Inc., Whitehouse Station, NJ, http://www.merck.com), and cyclophosphamide are the standard chemotherapy agents. The IRS has used therapeutic window studies to confirm the predictive nature of preclinical xenograft models and to identify several new single agents and combinations of agents with activity in high-risk patient groups. Despite these efforts, the outcome for these high-risk patients remains poor. The next generation of Children's Oncology Group studies will evaluate the efficacy of topoisomerase-I inhibitors and dose-compression therapy approaches. New advances in molecular characterization of tumors, including gene-expression analysis, may identify new therapeutic targets that can be exploited by expanded preclinical drug discovery efforts, and hold the promise of revolutionizing risk-based therapies.
Topics: Combined Modality Therapy; Humans; Rhabdomyosarcoma; Soft Tissue Neoplasms
PubMed: 16079319
DOI: 10.1634/theoncologist.10-7-518 -
American Family Physician Apr 2000Although cancer has an annual incidence of only about 150 new cases per 1 million U.S. children, it is the second leading cause of childhood deaths. Early detection and... (Review)
Review
Although cancer has an annual incidence of only about 150 new cases per 1 million U.S. children, it is the second leading cause of childhood deaths. Early detection and prompt therapy have the potential to reduce mortality. Leukemias, lymphomas and central nervous system tumors account for more than one half of new cancer cases in children. Early in the disease, leukemia may cause nonspecific symptoms similar to those of a viral infection. Leukemia should be suspected if persistent vague symptoms are accompanied by evidence of abnormal bleeding, bone pain, lymphadenopathy or hepatosplenomegaly. The presenting symptoms of a brain tumor may include elevated intracranial pressure, nerve abnormalities and seizures. A spinal tumor often presents with signs and symptoms of spinal cord compression. In children, lymphoma may present as one or more painless masses, often in the neck, accompanied by signs and symptoms resulting from local compression, as well as signs and symptoms of systemic disturbances, such as fever and weight loss. A neuroblastoma may arise from sympathetic nervous tissue anywhere in the body, but this tumor most often develops in the abdomen. The presentation depends on the local effects of the solid tumor and any metastases. An abdominal mass in a child may also be due to Wilms' tumor. This neoplasm may present with renal signs and symptoms, such as hypertension, hematuria and abdominal pain. A tumor of the musculoskeletal system is often first detected when trauma appears to cause pain and dysfunction out of proportion to the injury. Primary care physicians should be alert for possible presenting signs and symptoms of childhood malignancy, particularly in patients with Down syndrome or other congenital and familial conditions associated with an increased risk of cancer.
Topics: Bone Neoplasms; Central Nervous System Neoplasms; Child; Diagnosis, Differential; Humans; Kidney Neoplasms; Leukemia; Lymphoma; Neoplasms; Neoplasms, Muscle Tissue; Neuroblastoma; Wilms Tumor
PubMed: 10779255
DOI: No ID Found -
Current Oncology (Toronto, Ont.) Apr 2022Leiomyomas are a common type of benign soft tissue tumor arising from smooth muscle, most often occurring within females' genitourinary and gastrointestinal tract.... (Review)
Review
Leiomyomas are a common type of benign soft tissue tumor arising from smooth muscle, most often occurring within females' genitourinary and gastrointestinal tract. However, primary leiomyomas of the chest wall residing in the extra-pleural space are an extremely rare subset of leiomyomatous lesion presentation. We present a case of a fifty-two-year-old male who initially presented complaining of dyspnea worsening with exertion. Computed tomography imaging was performed showing an extra-pleural mass residing under the left sixth rib. Subsequent core needle biopsy and immunohistochemical staining were performed, and the definitive diagnosis of primary leiomyoma of the posterior mediastinal chest wall. Although extremely rare, this neoplastic condition should be included in your differential diagnosis when diagnostic imaging reveals a benign mass residing in the extra-pleural space, and subsequent biopsy specimens consist of smooth muscle fibers.
Topics: Female; Humans; Leiomyoma; Male; Middle Aged; Pleura; Soft Tissue Neoplasms; Thoracic Wall; Tomography, X-Ray Computed
PubMed: 35621630
DOI: 10.3390/curroncol29050240 -
BMJ Case Reports Oct 2016Mammary myofibroblastoma is a rare, benign, mesenchymal neoplasm that was first described in the breast in 1987. However, extra-mammary-type myofibroblastoma (MTMF) was... (Review)
Review
Mammary myofibroblastoma is a rare, benign, mesenchymal neoplasm that was first described in the breast in 1987. However, extra-mammary-type myofibroblastoma (MTMF) was not described until 2001 as a distinct entity. We report a case of extra-MTMF arising from the retroperitoneum, which was encountered in our clinic. We also completed a review of the literature using PubMed in patients with extra-MTMF.
Topics: Aged; Humans; Incidental Findings; Magnetic Resonance Imaging; Male; Neoplasms, Muscle Tissue; Psoas Muscles; Retroperitoneal Neoplasms; Tomography, X-Ray Computed
PubMed: 27742646
DOI: 10.1136/bcr-2016-217862 -
Annals of the Royal College of Surgeons... Jul 2017A rhabdomyomatous mesenchymal hamartoma (RMH) is a rare benign soft tissue tumour found in the face and neck region of children. A three-month-old male infant was...
A rhabdomyomatous mesenchymal hamartoma (RMH) is a rare benign soft tissue tumour found in the face and neck region of children. A three-month-old male infant was referred to our unit with a polypoid anal lesion that had been present from birth. Histopathological examination of the excised sample showed haphazard arrangement of mature striated skeletal muscle in among nerves and blood vessels, and immunohistochemistry confirmed the lesion to be a RMH. There was no evidence of recurrence during the follow-up period. This case emphasises two points: the unique histological characteristics associated with RMH and how this allows distinction from other differential diagnoses, and the consideration of RMH as a diagnosis in perianal lesions.
Topics: Anus Neoplasms; Hamartoma; Humans; Infant; Male; Rhabdomyoma
PubMed: 28660814
DOI: 10.1308/rcsann.2017.0115 -
Medicine Aug 2021Rectal inflammatory myofibroblastic tumor (IMT) is an extremely rare mesenchymal tumor characterized by a mixture of spindle-shaped myofibroblasts or fibroblasts and... (Review)
Review
RATIONALE
Rectal inflammatory myofibroblastic tumor (IMT) is an extremely rare mesenchymal tumor characterized by a mixture of spindle-shaped myofibroblasts or fibroblasts and inflammatory infiltration of lymphocytes and plasma cells. To date, only 8 cases of rectal IMT have been reported. Herein, we report an additional case of rectal IMT in a 28-year-old woman.
PATIENT CONCERNS
A 28-year-old woman presented with abdominal pain and hematochezia.
DIAGNOSES
Colonoscopy showed a 3.0-cm subepithelial tumor with central ulceration, covered by white exudate in the rectum. Rectal magnetic resonance imaging revealed a 4.0 × 3.0-cm-sized well-defined subepithelial tumor in the right wall of the rectum, with suspicious right perirectal fat infiltration.
INTERVENTIONS
Laparoscopic anterior resection was performed. Microscopic examination of the surgical specimen revealed bland-looking spindle cells intermingled with lymphoplasma cells. Immunohistochemistry and fluorescence in situ hybridization showed anaplastic lymphoma kinase positivity and anaplastic lymphoma kinase positivity rearrangement. Rectal IMT was confirmed based on histological, immunohistochemical, and fluorescence in situ hybridization findings. The patient was doing well without evidence of tumor recurrence 1 year after the surgery.
LESSONS
Rectal IMT, despite its rarity, should be considered in the differential diagnosis of rectal cancer. Second, an accurate histopathologic diagnosis and complete surgical resection can be the most important approaches to offer a chance for the cure of rectal IMT.
Topics: Adult; Female; Humans; Laparoscopy; Neoplasms, Muscle Tissue; Rectum
PubMed: 34414993
DOI: 10.1097/MD.0000000000027008 -
Modern Pathology : An Official Journal... Jan 2007Prostatic spindle cell lesions are diagnostically challenging and encompass a broad array of benign and malignant processes. A subset of these lesions arises only within... (Review)
Review
Prostatic spindle cell lesions are diagnostically challenging and encompass a broad array of benign and malignant processes. A subset of these lesions arises only within the prostate and generally represents entities that originate from the prostate epithelium or stroma, such as sclerosing adenosis, sarcomatoid carcinoma, stromal tumors of uncertain malignant potential (STUMP), and stromal sarcoma. Another subset of spindle cell tumors that involve the prostate are also found at other sites and include solitary fibrous tumor, leiomyosarcoma, and neural lesions among others. Finally, tumors may secondarily involve the prostate yet present as primary prostatic processes, as is evident with several cases of gastrointestinal stromal tumors (GIST). The utility of ancillary studies, including immunohistochemistry, is often limited and the main criteria for diagnosis are the morphologic findings by routine H&E stain. This review addresses the various entities that may present as spindle cell tumors within the adult prostate and discusses the functional aspects of the differential diagnosis of these lesions.
Topics: Adult; Carcinosarcoma; Diagnosis, Differential; Gastrointestinal Stromal Tumors; Humans; Leiomyoma; Leiomyosarcoma; Male; Neoplasms, Muscle Tissue; Prostate; Prostatic Hyperplasia; Prostatic Neoplasms; Rhabdomyosarcoma; Sarcoma; Sclerosis
PubMed: 17170745
DOI: 10.1038/modpathol.3800676 -
Revista de La Facultad de Ciencias... Sep 2022Primary skin leiomyosarcomas are infrequent neoplasms. They correspond to 2-3% of skin sarcomas and are most frequently located on the lower extremities, trunk and...
INTRODUCTION
Primary skin leiomyosarcomas are infrequent neoplasms. They correspond to 2-3% of skin sarcomas and are most frequently located on the lower extremities, trunk and genitals.
METHODS
We present a case of a 73-year-old man with a 4-month evolution of foreskin leiomyosarcoma. The lesion was biopsied for histopathological study with HE and immunohistochemistry with smooth muscle actin, specific muscle actin, CD34, p63 and S-100 (-).
RESULTS
We observed a leiomyosarcoma of high histological grade and mitotic count. It was positive by immunohistochemistry for smooth muscle actin, while the other markers were negative. Surgical limits were compromised so a reoperation with wide margins of healthy tissue was necessary.
CONCLUSION
The skin lesions should be removed all, without exception, since they can be neoplasms of variable biological behavior. The histological study must be complemented with immunohistochemistry to differentiate them from other neoplasms. For the prognosis, the histological grade, size, location and the possibility of resection with wide margins must be taken into account.
Topics: Actins; Aged; Foreskin; Humans; Immunohistochemistry; Leiomyosarcoma; Male; Prognosis
PubMed: 36149076
DOI: 10.31053/1853.0605.v79.n3.34628